Osteoporosis, bone & mineral

Nephrocalcinosis and kidney function in children and adults with X-linked hypophosphatemia: baseline results from a large longitudinal study

J Bone Miner ResPublished Cross-sectional study

Authors' conclusion

Nephrocalcinosis was observed in nearly one-quarter of children and more than one-third of adults with XLH. Further study is needed to better understand the predictors and long-term consequences of nephrocalcinosis, with surveillance for nephrocalcinosis remaining important in the management of XLH.

Study context

Population
Burosumab-naïve patients with X-linked hypophosphatemia (XLH) in burosumab clinical trials or the XLH Disease Monitoring Program (XLH-DMP); nearly all previously received conventional therapy (oral phosphate salts and active vitamin D)
Participants
196 vs 318
Exposure
Patients with vs without nephrocalcinosis
  • Childrenn = 196
  • Adultsn = 318
Outcome
Nephrocalcinosis on renal US
Follow-up
Baseline
Methodological detailsbaseline analysis of burosumab clinical trials and XLH Disease Monitoring Program
  • Baseline analysis of burosumab clinical trials and XLH Disease Monitoring Program

Main finding

As reported in the abstract

Observational data: associations, not effects.

Further findings

Reduced eGFR <90 mL/min/1.73 m² by nephrocalcinosis · baseline

Children with nephrocalcinosis
25%
Children without nephrocalcinosis
11%
Higher prevalence with nephrocalcinosis not observed in adults

Laboratory values in children with vs without nephrocalcinosis · baseline

Lower mean TmP/GFR with nephrocalcinosis
P < 0.05
Lower mean eGFR with nephrocalcinosis
P < 0.001
Higher mean serum calcium with nephrocalcinosis
P < 0.05

Laboratory values in adults with vs without nephrocalcinosis · baseline

Lower mean serum phosphorus with nephrocalcinosis
P < 0.01

Exploratory logistic regression: nephrocalcinosis vs other patient or disease characteristics

No significant associations
  • Exploratory

Parts of this Brief are withheld: the automatic checks could not confirm them against the abstract.

Original publication

Nephrocalcinosis and kidney function in children and adults with X-linked hypophosphatemia: baseline results from a large longitudinal study. J Bone Miner Res. Published 16 August 2024. doi:10.1093/jbmr/zjae127

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